Hirschsprung's disease in adolescents and adults

ABSTRACT

Hirschsprung's disease [HD] is rare in the adolescent and adult. It is often misdiagnosed cause of life-long refractory constipation. This study was undertaken to find out the best management strategy for HD in this specific age group. This retrospective study included 6 adolescent and 7 adult patients [10 males and 3 females] with proven tissue diagnosis of HD. These patients were treated between 1996 and 2005 at Tanta University Hospital and affiliated hospitals. The clinical data, diagnostic tools, details of surgical treatment and outcome were analyzed. The patient's ages ranged from 13-27 years [mean 21 +/- 3 years]. The aganglionic segments were confined to the rectum in nine patients and included the distal part of sigmoid in four. All patients presented with chronic constipation requiring enemas, cathartics, and multiple hospital admissions for management. The diagnosis was confirmed by barium enema and full-thickness rectal biopsy. Four patients had anorectal manometry. The definitive procedures were two-stage endorectal pull through [ERPT] [n=5], primary ERPT [n=2], primary transanal ERPT [n=2], ERPT after failed anorectal myectomy [n=2 patients], Duhamel after failed myectomy [n=1], and low anterior resection and myectomy [n=l]. These operations were complicated with anastomotic leak in 2 patients [15.4%], anal stricture [n=1] and cuff abscess [n=1]. Three patients [23.1%] have partial incontinence, while the other 10 patients have good functional results. 1. The diagnosis of HD should always be suspected in the context of chronic constipation in adolescents and adults; 2. although primary pull through is feasible after prolonged bowel preparation, a two -stage approach is more technically easier and safer; 3. Despite a relative higher incidence of complications, the functional results after surgery in adult patients is comparable to those achieved in children